UHDRS

The UHDRS is a research tool which has been developed by the HSG to provide a uniform assessment of the clinical features and course of HD. The UHDRS has undergone extensive reliability and validity testing and has been used as a major outcome measure by the HSG in controlled clinical trials.

UHDRS '99

UHDRS '99 represents an update and expansion of the UHDRS to augment the utility and applicability of this research tool. In 1999 a team of HD researchers enhanced all of the components of the UHDRS. In 2005 the Behavioral Assessment section was further clarified and refined based on research experience.

HOW TO OBTAIN PERMISSION TO USE THE UHDRS

The Unified Huntington Disease Rating Scale and revised UHDRS '99 and accompanying manual created by the HSG members are the copyrighted intellectual property of the HSG (hereinafter collectively referred to as the, "UHDRS"). The UHDRS, case report forms, and other related materials are copyrighted by the HSG. Individuals, organizations, firms, or other entities seeking to use these tools and/or materials must obtain prior written permission from the HSG. License fees may apply.

Please complete the UHDRS Request formto obtain permission and approval for use of the UHDRS scales or UHDRS Motor Training DVD.

Please note that there are certain non-HSG components incorporated within the UHDRS that are not the intellectual property of the HSG, and therefore, you will be required to seek and obtain separate permissions from the owners of these scales.

These include the following:

• Symbol Digit Modalities Test (SDMT) – Western Psychological Services owns this test. The testing form based on the original SDMT manuscript must be purchased for use. If an alternative version of the SDMT is used, permission must be obtained from WPS and a fee for use must be paid to WPS.
• Stroop and Verbal Fluency - these scales appear to be in the public domain, however, you need to obtain permission for use from Psychological Assessment Resources, Inc.
• TFC – Permission for use is needed for this assessment tool from the author Ira Shoulson, MD.

UHDRS NATURAL HISTORY DATABASE

The UHDRS Natural History Database is a compilation of more than 8,000 individuals. The database includes individuals affected by or immediately at risk for HD. HSG researchers have used this valuable cohort to investigate research questions. The following are two of many research publications using this cohort. Please click on HSG publications for additional manuscripts based on the UHDRS Natural History Database.

Factors associated with the rate of functional decline were evaluated using the UHDRS natural history database in a large HD cohort followed longitudinally for up to four years. A full report was published by The Huntington Study Group (Marder K, primary author). Rate of functional decline in Huntington’s disease. Neurology 54:452-458, 2000.

Early identification of clinical changes in HD may be of critical importance for the design and implementation of clinical trials to slow the progression of the disease. A full report of this paper was published by: The Huntington Study Group (Paulsen JS, primary author). Clinical markers of early disease in persons near onset of Huntington’s disease. Neurology 2001;57:658-662.